An Interesting Plan

Ezra's counts have been stable for the past two weeks and there thankfully has been no sign of the auto-antibodies. The doctors have slowed down the steroid wean now that Ezra is on a lower dose and the chance of a flare-up is higher. Frustrating that we are going so slowly, but it's better than a flare-up. His liver levels have been close to normal over the past couple of weeks for the first time in longer than we can remember. Another T cell check is cooking in the lab. We have zero expectations for his T cell numbers, but are hopeful as always for an increase. 

Ezra's doctors have come up with an interesting plan. They want to try and vaccinate him. Typically, they would not try to vaccinate until he reaches a certain number of T cells. The reasons are complicated to explain, but basically, he has now reached a dosing on the steroids where they think he may be capable of having an immune response. The reason for trying now is that it has been almost two and a half months since his last dose of IVIG. They can only check for an antibody response to vaccination when time has passed from the last IVIG as IVIG provides the antibodies that Ezra hasn't been able to make on his own. Ezra's IgG levels have unfortunately been dropping due to the immune suppression and he is at the point when we would normally give him IVIG. Now is an ideal time to hold off on the IVIG to do this vaccine challenge because the germy season has not yet hit. The hope is also that his body will kick in on the IgG production in the meantime now that the levels are low. 

Ezra will be given the vaccine at the end of the week, they will get a baseline, and then test for antibodies two weeks later. If he makes an antibody response, it won't necessarily change anything about his current situation because he remains at risk for life-threatening infection until he has more T cells. But it would be HUGE if he is able to make antibodies in response to the vaccine. There are a lot of issues with Hyper IgM, but the primary issue is the inability to make antibodies. If he responds to the vaccine, then we know the few T cells he does have are working and we are on our way to a cure. If he doesn't respond, then his immune system just isn't ready yet. It's worth a shot ;)

As everyone is returning to school, Ezra had the last of his weekly visits to "school" this week. As you can see, he loved every minute of it. We had decided to do the second transplant when we did with the goal of getting Ezra healthy in time to start kindergarten. We've had to come to grips with the reality that kindergarten is not happening - at least, not yet. It's heartbreaking, but at this point, all we want is a healthy Ezra. We are bringing kindergarten to Ezra - tomorrow he starts at home with a wonderful kindergarten teacher, in addition to his beloved teachers and therapists. 

Team Ezra is off to a great start! Team Ezra T-shirts are now available! Check out these awesome, Rube Goldberg theme purple (Ezra's favorite color) shirts: http://www.goodthreads.com/SharedDesign/TeamEzra2.design
$5 of every shirt goes to Gift of Life/Team Ezra fundraising for the Walk for Life. Special thanks to Jodie Hein and Ken Bowser for designing these fantastic shirts! Show your Team Ezra pride!

Day +365: One Year - A Celebration of Life

One year. A year like no other. Looking back on the past year, it feels like it can be divided by all the challenges Ezra has faced. First, round two of high dose chemo, transplant, engraftment and the unspeakable suffering of that time. Next, home and the horrific pain that Ezra suffered, which made him cry all day, as he sat covered in hot packs in a fog from pain medication. Then, just as the pain resolved, came skin GVHD and the round of immune-suppressing steroids used to stop it. Next up, parainfluenza virus and liver issues. And just when he had gotten over the virus and stopped the steroids, the auto-antibodies against his red blood cells, platelets and neutrophils started their vicious attack. Back in the hospital for the month of March with this frightening turn of events, a problem that continues to plague him and drugs that continue to prevent his new immune system from growing.

We still do not know how we ever mustered the strength to walk our baby back into the hospital and subject him once again to the nightmare of transplant. It was solely our belief that transplant was his best chance at a long, healthy life. And that we owed it to him to try and give him that life.

While there is much of transplant that we try not to remember, there is too much that we will never forget. The moment of transplant. Eyeing that impossibly small syringe and not believing that small amount of cells could save our son's life and grow into a new immune system. Everything - all of our lives - hanging on a minuscule amount of cells. At that moment, remembering how hard it was to breathe behind those masks - they make you feel like you're hyperventilating. And maybe we were. But the mask did not stop us from covering Ezra's chemo-soured head with kisses and wondering when he would once again smell like baby shampoo.

We have never gone into great detail as to the horrors chemo and the transplant process inflicted on Ezra's little body, especially the second time around. Suffice to say that it was horrible. But what is amazing, is the way that his body has healed and dealt with all the changes in the past two and a half years of two transplant journeys. From pre-transplant to the horrors of transplant to post-transplant weight loss and hair loss to the bloating of steroids to the still puffy, but not as puffy of today - it is breathtaking to see how his body has changed. We have been trying to teach Ezra to appreciate the way his body can heal and to take comfort in his body's incredible ability to heal.

Our hope that Ezra will someday be cured would be impossible without Ezra's miracle needle-in-a-haystack donor. Our hope when we had none. We owe everything to her and we hope she knows how much she means to us. We feel so much gratitude that a cure even exists, as difficult as it may be, and that Ezra's doctors have gotten him this far.

In celebration of Ezra's life, we are forming Team Ezra for the Gift of Life Bone Marrow Foundation Walk for Life taking place on Sunday, October 26 in Paramus, New Jersey. The event is a 5K walk or run and includes a donor-recipient meeting, kids area and entertainment. All net proceeds from the walk go to adding more donors to the registry. Please join us in honoring Ezra and helping others have a second chance at life. Register for Team Ezra at http://www.giftoflife.org/njwalk/teamezra. If you can't make the walk please consider donating to Team Ezra or to our family fundraising page 

http://gol.convio.net/site/TR/WalkForLife/General?px=1293644&pg=personal&fr_id=1150

We are also in the process of designing an awesome Team Ezra shirt - stay tuned!

We celebrated the one year transplant anniversary over the past few days with cake and balloons and a special shirt for Ezra that says "I survived TWO stem cell transplants" (who knew they made such shirts? And in kid sizes?). Our journey for a cure is sadly nowhere near over. We enter year two post-transplant with a certain amount of fear of what may come, but mainly we are hopeful. Hopeful that this year may be the year that Ezra is cured. 

Ezra has a daily calendar that he fills out each day with his teacher. It has a spot for feelings. Everyday Ezra chooses "Happy" because, as he says, "I am always happy." This is what we hang onto more than anything - our happy little boy who leads us through the darkest of days so that all we see is light. 

Days +354 to +360: We Will Get There

We got back the results of Ezra's monthly T cell check and unfortunately Ezra's T cells decreased this month. The T cells are not as far down as they were in June, but it's enough movement in the wrong direction. The decrease in T cells is likely from the increase in steroids following Ezra's auto-antibody flare-up in June. After giving us the results, Ezra's doctor reassured us that "we will get there." We hold on tight to the belief that we will indeed get there. When and at what cost are the unknowns. We always say we're ok with this process taking a long time - after all, a life saving treatment shouldn't be easy - as long as we are moving forward. It is falling backwards, especially with losing precious T cells, that hurts. 

Ezra's toe is still a little red, but improved. We are continuing with the antibiotics for a bit longer and will go from there. His hemoglobin was down this week, but one of the indicators of red blood cell destruction was normal, so the doctors weren't too concerned. We are sitting tight on the steroids for this week and then hopefully be able to wean again next week. 

One piece of positive news: the platelet antibody screen came back negative. As of now, Ezra is testing negative for antibodies for all three cell lines! A negative screen doesn't mean that the antibodies are totally gone, but it is a sign that things are quieting down. They sent out another red blood cell antibody screen test on Friday because as we go down on steroids, the antibodies can flare-up again. We are so hoping these tests stay negative. 

While the summer is great for us because we can do so much outside, it is also challenging to go to any outdoor public places because so many more people are around in the summer. We did make a great discovery to keep Ezra busy and challenged - mini golf. We discovered a great mini golf place close-by. We are able to go at quiet times and keep distance from the other players. Ezra loves it and even hit a crazy hole-in-one! Last year on this day, Ezra was on Day -5 of chemo in preparation for transplant number two and was already very ill. So grateful to be breathing fresh air far away from the gray of the hospital. 

Days + 342 to +353: The Revolving Door of Issues

Last week, Ezra's platelet count had dropped again. Just as the red blood cells had come under control, of course his platelets needed to take their turn again and cause problems. The doctor was planning to see his counts this week and then give another round of high dose IVIG. We were feeling frustrated at how difficult it has been to get these auto-antibodies under control. 

Then we showed up to clinic on Friday and his platelets had bounced up nicely and are back in the normal range! A pleasant surprise that we will gladly accept. The doctor wanted to do another check of the platelet antibodies, which we haven't done for a while because it requires a tremendous amount of blood. It will be interesting to see what this test shows. 

Because there always has to be something, over the weekend, one of Ezra's big toes developed redness around the toe nail. It seems to be infected. He started antibiotics and foot soaks. We will be watching it closely. We are hoping the infection clears up quickly and doesn't cause problems with his counts, which it may do. Here we go yet again.

The doctor also sent out another T cell count check on Friday (yeah, we're not sure how he has any blood left). Ezra's total lymphocyte count, which includes T cells and other types of cells, has been low, so we have no expectations of what this month's T cell count will show. Please prayers for a T cell increase!

As we approach the one year anniversary of Ezra's transplant on August 23, there are a lot of intense memories for us associated with the month of August.  This week marks one year from when Ezra was re-admitted to start high dose chemotherapy for the second transplant. Five years ago this past week, a 5 month old Ezra was admitted to our local hospital with labored breathing and would soon be escalated to intensive care. For the first time five years ago, we heard the name "Hyper IgM Syndrome," and life forever changed. August may always be a difficult month for us, but we have plans to change it into a month when we will also always celebrate life and hope. 

Days +334 to +341: Got To Keep It Up

Just a quick update to let everyone know that Ezra's counts were once again good this week. Ezra's doctor called his counts "beautiful." His hemoglobin was up and all the other numbers were normal. The only issue, aside from the overarching issue of Ezra needing to grow a new immune system, is his liver function. His liver levels have unfortunately increased again. The doctor believes that it is from the increase in steroids. For now, the plan is to wait and see if the levels decrease as we go down on the steroids. There always has to be something of concern on this crazy journey. 

Ezra also had an eye exam to check his eyes for side effects from his two transplants. They are also going to be watching his eyes closely for cataracts, which can be a side effect of steroids. The doctor said it's too soon to see cataracts from the steroids, but for now, Ezra's eyes got an "A++."

We really, really need a period of calm and good counts to get Ezra off the steroids and immunosuppressant. We went down again by a small amount on the steroids, but it's going to take some time before he's even back to where he was on the wean before this latest flare up. Getting off these drugs is the key to allowing Ezra's donor cells to build him the new immune system he so desperately needs. We know those donor cells are there, waiting to be allowed to grow. We can't wait for them to break Ezra free of his life of isolation, and pray that it is only a matter of time. 

Days +327 to +333: A Good Week

We finally have good news to report this week. First, Ezra's counts were great. His hemoglobin was normal and one of the measures of red blood cell destruction was also completely normal for the first time since he developed the antibodies. Second, the latest test of the strength of the red blood cell antibodies came back negative, showing no antibodies. Huh? Yep. This happened once before when he tested negative for the antibody and then two weeks later tested positive again. The doctor explained that the antibodies are probably there, but not at measurable levels at the moment. The flare-up that Ezra has been experiencing over the past three weeks was likely caused by a combination of the rhinovirus and the decrease in the steroids. Whatever it is, and even if the test comes back positive again two weeks from now, it is certainly good to have a period of calm and healing. 

Even more surprising - Ezra's T cells went up! They are back to where they were in May when they first dropped. The level is still far from where it was before he went on steroids at the end of February and still very far from where he needs to be, but he is headed in the right direction. Ezra's doctor was very surprised at this increase. She did not expect a T cell increase with the current steroid dose. That's Ezra for you - always doing things his own way. 

The question is - can he keep these T cells or is he going to lose them again because of the steroids? The doctor thinks he will keep them provided we are able to continue weaning the steroids. Despite all this good news, the doctor lowered the steroid dose only by the smallest of increments again this week. She is proceeding with caution for now to avoid another flare up. So it remains to be seen if Ezra will be able to maintain this T cell progress. 

By now we know all too well that we can lose this progress as quickly as we gained it. There have been way too many ups and downs on this journey for us to feel like Ezra is at all safe. Despite the number of times we've been knocked down, we are still managing to feel optimistic from these latest numbers. A little hope goes a long way. 

The other exciting news of the week is that Ezra had his first day of school in school! Ezra's teachers came up with the great idea of having Ezra do his home schooling at one of the schools that is closed for the summer so that he can get a sense of what it is like to sit in a classroom and what a school looks like. They had one of the preschool rooms thoroughly cleaned, air conditioners cleared out, and a sign hung on the door welcoming Ezra to school. The look on his face when his teachers told him that he was going to the school was indescribable. He was just so happy. 

Class started with the Pledge of Allegiance. Ezra was very taken by the smart board in the classroom. He was so focused on his school work - it was amazing to see. He will continue to go to the school once a week for the summer. We are very grateful for the creativity and determination of his teachers who arranged for this incredible opportunity. Our hope is that when the day comes for him to attend "real" school with other children that he will be as excited and happy to attend as he is with his class of one. 

Days +320 to +326: A Mixed Bag

It was another week of up and down counts. Ezra's body is still struggling to get back on track. The main issue at the moment seems to be red blood cell destruction. His platelets thankfully remained in the normal range this week. His hemoglobin, on the other hand, has been up and down. At the end of the week, his hemoglobin had improved. However, one of the measures of red blood cell destruction was higher, reflecting that his body is working hard to replace red blood cells that are being destroyed. The doctor was actually happier with this scenario than when his hemoglobin was lower because it means that his body is able to compensate for the destruction. 

The doctor decided to lower his steroid dose by the smallest increment and only on alternate days. We are really hoping that this minor decrease doesn't affect his counts and that we can get back to weaning the steroids. This weekend, we already saw a regression in Ezra's strength and endurance from the higher steroids. Steroids just stink. 

We did receive the results of Ezra's latest chimerism test and thankfully, he is still 100% donor on all cell lines. We take comfort in knowing that when the day comes when he is off the steroids and immune suppressants, Ezra will have donor cells ready to build him a new, strong immune system (we hope). 

Ezra started his summer school session this week. To match his 100% donor cells, he got 100% on his first ever spelling test (we're allowed a little bragging, right?)! Maybe his donor is a good speller. :)